The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases.
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Long non-coding RNAs in nervous system function and diseaseFUS-mediated regulation of alternative RNA processing in neurons: insights from global transcriptome analysisPhenotypic Heterogeneity of Monogenic Frontotemporal DementiaThe function of RNA-binding proteins at the synapse: implications for neurodegenerationA loss of FUS/TLS function leads to impaired cellular proliferationEarly lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif.Non-coding RNA in neural function, disease, and agingDistinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusionsGene expression responses to FUS, EWS, and TAF15 reduction and stress granule sequestration analyses identifies FET-protein non-redundant functions.Prions, amyloids, and RNA: Pieces of a puzzle.RRM domain of ALS/FTD-causing FUS characteristic of irreversible unfolding spontaneously self-assembles into amyloid fibrils.Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLSA yeast model of FUS/TLS-dependent cytotoxicity.FUS immunogold labeling TEM analysis of the neuronal cytoplasmic inclusions of neuronal intermediate filament inclusion disease: a frontotemporal lobar degeneration with FUS proteinopathy.An aggregation sensing reporter identifies leflunomide and teriflunomide as polyglutamine aggregate inhibitors.FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations.The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease.Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granulesKnockdown of the Drosophila fused in sarcoma (FUS) homologue causes deficient locomotive behavior and shortening of motoneuron terminal branches.The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrumNeurodegeneration the RNA wayLentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteinsGenetics of amyotrophic lateral sclerosis: an update.RNA-mediated toxicity in neurodegenerative diseaseIdentification of an RNA Polymerase III Regulator Linked to Disease-Associated Protein Aggregation.TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration.Proteostasis and movement disorders: Parkinson's disease and amyotrophic lateral sclerosis.RNA-binding proteins with prion-like domains in ALS and FTLD-U.TARDBP and FUS mutations associated with amyotrophic lateral sclerosis: summary and update.Stress granules in neurodegeneration--lessons learnt from TAR DNA binding protein of 43 kDa and fused in sarcoma.Protein aggregation in amyotrophic lateral sclerosis.TDP-43 pathology in polyglutamine diseases: with reference to amyotrphic lateral sclerosis.The role of FUS gene variants in neurodegenerative diseases.Aggregation of FET Proteins as a Pathological Change in Amyotrophic Lateral Sclerosis.Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis.Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models.The seeds of neurodegeneration: prion-like spreading in ALSIntracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations.Prevalence of Huntington's disease gene CAG repeat alleles in sporadic amyotrophic lateral sclerosis patients.Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations.
P2860
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P2860
The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases.
description
2009 nî lūn-bûn
@nan
2009年の論文
@ja
2009年学术文章
@wuu
2009年学术文章
@zh
2009年学术文章
@zh-cn
2009年学术文章
@zh-hans
2009年学术文章
@zh-my
2009年学术文章
@zh-sg
2009年學術文章
@yue
2009年學術文章
@zh-hant
name
The RNA-binding protein FUS/TL ...... ein in polyglutamine diseases.
@en
The RNA-binding protein FUS/TL ...... ein in polyglutamine diseases.
@nl
type
label
The RNA-binding protein FUS/TL ...... ein in polyglutamine diseases.
@en
The RNA-binding protein FUS/TL ...... ein in polyglutamine diseases.
@nl
prefLabel
The RNA-binding protein FUS/TL ...... ein in polyglutamine diseases.
@en
The RNA-binding protein FUS/TL ...... ein in polyglutamine diseases.
@nl
P2093
P1476
The RNA-binding protein FUS/TL ...... ein in polyglutamine diseases.
@en
P2093
Hiroshi Doi
Nobuyuki Nukina
Shigeru Koyano
Yoshiyuki Kuroiwa
Yume Suzuki
P304
P356
10.1016/J.NEURES.2009.10.004
P577
2009-10-13T00:00:00Z